Inflammation and repeated infections in CGD: two sides of a coin.
Inflammation and repeated infections in CGD: two sides of a coin.
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DOI：
10.1007/s00018-011-0834-z
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发表时间：
2012-01
影响因子：
8
通讯作者：
Lutter, Rene
中科院分区：
文献类型：
--
作者：
Kuijpers, Taco;Lutter, Rene
慢性肉芽肿性疾病（CGD）是一种罕见的先天性免疫缺陷，约1/250，000的个体。它是由一个深刻的缺陷，在爆发的氧气消耗，通常伴随着吞噬作用在所有骨髓细胞（中性粒细胞，嗜酸性粒细胞，单核细胞和巨噬细胞）。这种"呼吸爆发"涉及分子氧催化转化为氧自由基超氧化物，这反过来又产生过氧化氢，次氯酸和羟基自由基。这些氧衍生物在杀死致病细菌和真菌方面起着关键作用。由于未能激活其吞噬细胞中的呼吸爆发，大多数CGD患者患有严重的复发性感染和可能导致肉芽肿性病变的原因不明的长期炎症反应。两者都可能导致严重的器官功能障碍，这取决于所涉及的组织。成功治疗CGD需要采取预防措施以及快速（侵入性）诊断程序。造血干细胞移植可能是一些患者的一个重要选择。
Chronic granulomatous disease (CGD) is an uncommon congenital immunodeficiency seen approximately in 1 of 250,000 individuals. It is caused by a profound defect in a burst of oxygen consumption that normally accompanies phagocytosis in all myeloid cells (neutrophils, eosinophils, monocytes, and macrophages). This “respiratory burst” involves the catalytic conversion of molecular oxygen to the oxygen free-radical superoxide, which in turn gives rise to hydrogen peroxide, hypochlorous acid, and hydroxyl radicals. These oxygen derivatives play a critical role in the killing of pathogenic bacteria and fungi. As a result of the failure to activate the respiratory burst in their phagocytes, the majority of CGD patients suffer from severe recurrent infections and rather unexplained prolonged inflammatory reactions that may result in granulomatous lesions. Both may cause severe organ dysfunction depending on the tissues involved. Preventive measures as well as rapid (invasive) diagnostic procedures are required to successfully treat CGD. Hematopoietic stem cell transplantation may be a serious option in some of the patients.
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影响因子：
20.3
作者：
Matute, Juan D.;Arias, Andres A.;Dinauer, Mary C.
通讯作者：
Dinauer, Mary C.
DOI：
10.1084/jem.189.9.1363
发表时间：
1999-05-03
期刊：
The Journal of experimental medicine
影响因子：
--
作者：
Munn DH;Shafizadeh E;Attwood JT;Bondarev I;Pashine A;Mellor AL
通讯作者：
Mellor AL
DOI：
10.1084/jem.20082767
发表时间：
2009-06-08
期刊：
The Journal of experimental medicine
影响因子：
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作者：
Minegishi Y;Saito M;Nagasawa M;Takada H;Hara T;Tsuchiya S;Agematsu K;Yamada M;Kawamura N;Ariga T;Tsuge I;Karasuyama H
通讯作者：
Karasuyama H
影响因子：
5.1
作者：
GALLIN, JI;BUESCHER, ES
通讯作者：
BUESCHER, ES
DOI：
10.1073/pnas.0604571103
发表时间：
2006-08-22
影响因子：
11.1
作者：
Gelderman, Kyra A.;Hultqvist, Malin;Holmdahl, Rikard
通讯作者：
Holmdahl, Rikard